Hamartomas are tumours composed of mesenchymal tissues such as cartilage, fat, connective tissue and smooth muscle and can be found in virtually any organ system. These masses commonly develop sporadically, but are also seen in certain syndromes such as tuberous sclerosis or Carney triad. While their imaging appearance varies depending on the organ they arise from, findings are usually unique and a diagnosis can be confidently made. Radiologists must be aware of the clinical and imaging presentations of these lesions with the particular goal of avoiding unnecessary studies or invasive procedures. Furthermore, knowledge of common syndromic entities is crucial, as the radiologist may be the first to suggest the diagnosis.

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IntroductionThe rare variant of renal epithelioid/pleomorphic angiomyolipoma has been reported in approximately 120 cases. One of the most important characteristics to differentiate these tumors from other renal cell neoplasms is their typical reactivity to premelanosome antigens. If such a tumor does not stain for HMB-45 or Melan-A, a specific diagnosis of epithelioid pleomorphic angiomyolipoma cannot be made with certainty.Case presentationWe present here what is, to the best of our knowledge, the first case of epithelioid/pleomorphic angiomyolipoma of the kidney in a 50-year-old Caucasian man with no history of tuberous sclerosis, and with a tumor marker profile negative for several premelanosome antigens. The tumor was composed of sheets of pleomorphic, round to polygonal epithelioid cells with prominent eosinophilic cytoplasm, large nuclei, many multinucleated, and very prominent nucleoli. There were prominent vessels and rare interspersed smooth muscle fibers, but adipocytes were not identified. A tumor marker profile showed tumor cell reactivity for CD68, calponin and focally for CD10. Intervening smooth muscle was reactive with smooth muscle actin. The tumor lacked reactivity for melanin-associated antigens HMB-45 and Melan-A, and for CD31, pan-cytokeratin (AE1/3) and desmin. Electron microscopic examination of tumor cells confirmed the presence of premelanosome-like granules.ConclusionsBased on the characteristic microscopic appearance of this tumor, and its overall tumor marker profile, we concluded this was a renal epithelioid/pleomorphic angiomyolipoma with a negative premelanosome antigen phenotype.

A wide variety of fat-containing entities occur in and near the heart. These findings are often encountered by radiologists and may be incidental or the reason for the patient's clinical presentation. Cross-sectional imaging helps to characterize the extent of these lesions and to formulate a differential diagnosis, which varies by lesion location, imaging features and patient demographics. The purpose of this pictorial essay is to familiarize radiologists with these fat-containing lesions and to help avoid misdiagnosis and errors in management. This pictorial review will discuss the normal fatty structures in and around the heart. A range of common and uncommon fat-containing lesions will then be reviewed based upon lesion location.

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